The 'CREST' syndrome. Comparison with systemic sclerosis (scleroderma)

Velayos, Edward E.

doi:10.1001/archinte.139.11.1240

Cited by 47 publications

(24 citation statements)

References 10 publications

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“…Clinical and laboratory data are collected at the first visit to the Johns Hopkins Scleroderma Center (JHSC) and then prospectively ascertained in an ongoing longitudinal database among those with follow-up visits to the Center. Each patient satisfied one of the following three criteria: [A] ACR criteria for scleroderma (15), [B] 3 or more of the 5 features of the CREST syndrome (16, 17), or [C] the combination of definite Raynaud phenomenon, abnormal nailfold capillaries and a SSc-specific autoantibody (18). A total of 2481 patients with SSc were enrolled in the database from 1990 until 2009, among which 2300 had a recorded date of SSc onset and comprehensive assessments, and therefore were included in the present analyses.…”

Section: Methodsmentioning

confidence: 99%

Late-age Onset Systemic Sclerosis

Manno

Wigley²,

Gelber³

et al. 2011

J Rheumatol

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OBJECTIVE-Although patients who develop scleroderma (SSc) later in life (≥ 65 years) may express the entire clinical spectrum of disease, we hypothesize that patients with late-age onset incur a different risk for specific organ manifestations of disease compared to those with youngerage onset SSc.METHODS-In total, 2300 SSc patients were evaluated between 1990-2009 and reviewed from a university-based Scleroderma Center cohort. Demographic profile, SSc subtype, autoantibody status, Medsger severity scores, pulmonary function tests, echocardiography, and right heart catheterization parameters were compared between late-age versus younger-age onset patients.RESULTS-Overall, 2084 (91%) patients developed SSc prior to age 65; whereas 216 (9%) were ≥65 years. Late-age onset patients had a significantly higher proportion of anti-centromere antibodies (42% vs 27%; p=0.001) compared to younger-age onset. Risk of pulmonary hypertension (OR 1.77; 95%CI 1.00, 3.12), muscle weakness (OR 1.85; 95%CI 1.30, 2.64), renal impairment (OR 2.83; 95%CI 1.98, 4.04) and cardiac disease (OR 2.69; 95%CI 1.92, 3.78) was greater among those with late-age onset SSc; although risk of digital ischemia (OR 0.64; 95%CI 0.47, 0.86) was reduced. The cumulative incidence of pulmonary hypertension at 5 years was greater among those with late-age (9%) compared to younger-age (2.5%) onset SSc (log-rank, p<0.001).CONCLUSION-These findings suggest that older SSc patients are at greater risk for pulmonary hypertension, renal impairment, cardiac disease, and muscle weakness. Awareness of the distinct risk for specific organ manifestations in SSc, in particular pulmonary hypertension, should guide the care of older SSc patients whose disease begins after age 65 years.

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Section: Methodsmentioning

confidence: 99%

Late-age Onset Systemic Sclerosis

Manno

Wigley²,

Gelber³

et al. 2011

J Rheumatol

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“…6 Patients were classified as having the CREST syndrome if any three of the following aetiologies were present: subcutaneous calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly or telangiectasia. 7 This investigation was approved by our institutional review board.…”

Section: Clinical Datamentioning

confidence: 99%

A case-control study of the pathology of oesophageal disease in systemic sclerosis (scleroderma)

Hummers

Ravich

et al. 2006

Gut

119

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Background: Atrophy of the smooth muscle layers of the muscularis propria characterises oesophageal involvement in systemic sclerosis (scleroderma). The aetiology of this atrophy and of the resultant oesophageal dysfunction is unknown. Objectives: To examine oesophageal tissue for evidence of fibrosis, vascular disease, inflammatory reactions and neural abnormalities to determine the possible causes of this disease process. Methods: A case-control survey was conducted using oesophageal tissue from 74 scleroderma cases and 74 age, race and sex-matched controls from our autopsy files. Histological evidence of oesophageal muscle atrophy was correlated with the degree of vascular changes, inflammatory infiltration, fibrosis, abnormalities of the myenteric plexus and reduction of interstitial cells of Cajal (ICC) using a predesigned semiquantitative descriptive method. Results: Smooth-muscle atrophy was found in 94% of scleroderma cases, and in 5% of controls (p,0.001). Atrophy was evident in the circular smooth muscle in 93% of cases, and in the longitudinal smooth muscle in 66% of cases. Intimal proliferation of arterioles was found in 38% of cases and in 5% of controls (p,0.001), but was not associated with smooth-muscle atrophy (p = 0.29). Despite these vascular changes, there was no evidence of compromised perfusion, such as findings suggestive of acute ischaemic necroses. Minimal cellular infiltrates were seen in the myenteric plexus in 82% of cases and in 92% of controls (p = 0.091). ICC were found in fewer numbers in areas of atrophic smooth muscle compared with adjacent normal smooth muscle in selected scleroderma cases. Conclusion: The pathological findings of oesophageal lesions in scleroderma seem inconsistent with either an ischaemic or an inflammatory process. The loss of circular and longitudinal smooth muscle in the distal scleroderma oesophagus may represent loss of normal neural function followed by secondary tissue atrophy, or may be a primary smooth muscle lesion.

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“…It was only in 1973 that esophageal dysmotility was added to complete the acronym CREST when Frayha et al 15 reported a high frequency of esophageal involvement in 25 patients with the CRST syndrome. Thus, the CRST and CREST syndromes are closely related disorders that belong in the clinicopathologic spectrum of progressive systemic sclerosis (PSS), or scleroderma.9 9 Earlier reports prematurely emphasized the prolonged and benign clinical course of CRST and CREST syndromes,4,5,14 although they were referring to the duration of one of the components, Raynaud's phenomenon. More recent comparative studies 6-12 have largely corrected this misconception by pointing out that although muscle, skin, and pulmonary involvements were greater in PSS than in CREST, there were no clinically significant differences between PSS and CREST for other visceral involvement, including gastrointestinal, renal, and cardiovascular disorders.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Hypertension in the CREST Syndrome: Variant of Systemic Sclerosis (Scleroderma) — A Case Report

Lie

1989

Angiology

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The CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) is a variant but not a benign form of scleroderma. Pulmonary hypertension without pulmonary fibrosis appears to be more prevalent in the CREST than in scleroderma patients. Moreover, pulmonary hypertension in CREST may be rapidly progressive and a cause of sudden death from severe cor pulmonale.

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The 'CREST' syndrome. Comparison with systemic sclerosis (scleroderma)

Cited by 47 publications

References 10 publications

Late-age Onset Systemic Sclerosis

Late-age Onset Systemic Sclerosis

A case-control study of the pathology of oesophageal disease in systemic sclerosis (scleroderma)

Pulmonary Hypertension in the CREST Syndrome: Variant of Systemic Sclerosis (Scleroderma) — A Case Report

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